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Long-term outcome and quality of life after the Swenson procedure for Hirschsprung's disease
Long-Term Outcome and Quality of Life After the Swenson Procedure for Hirschsprung’s Disease By YuZuo Bai, Hui Chen, Jing Hao, Ying Huang, and WeiLin Wang Shenyang, China
Background/Purpose: The aim of this study was to investigate long-term outcome and quality of life after the Swenson operation for rectosigmoid Hirschsprung’s Disease (HD). Methods: Forty-five patients who underwent the Swenson procedure for HD underwent follow-up for 8 to 16 years. Long-term outcome and quality of life were assessed by interviews and questionnaires including scoring systems. Forty-four healthy children with similar age, sex, and education level distributions used as controls. Results: In 45 patients, 23 (51.1%) had bowel dysfunction. Seventeen patients (37.8%) suffered from fecal soiling. According to the clinical bowel function scoring system, the patients’ scores (7.6 ⫾ 2.1) were significantly lower than those of the controls (11.4 ⫾ 0.6; P ⬍ .05). Because of poor fecal continence, 25 patients (55.7%) had to restrict their foods. School absence occurred in 6 (13.3%) patients. Seven patients (15.6%) had problems in peer relationships. According to the Quality-of-Life Scoring Criteria, 86.7% patients had good or fair quality of life. The patients’ scores (7.7 ⫾ 2.9)
W
ITH MANY ADVANCES in the surgical management of Hirschsprung’s disease (HD) in the modern era, the postoperative results of surgically treated HD generally are satisfactory.1-5 However, some patients continued to have persistent bowel dysfunction including incontinence, constipation, and enterocolitis. Several reports showed less favorable findings. The long-term outcome of surgery for HD is variable.1-16 In addition, the majority of long-term follow-up studies have concentrated only on the functional outcome. Although this is important, little is known about the quality of life in patients after surgical treatment for HD. The aim of this study was to investigate the long-term clinical outcome and quality of life in patients with surgical correction of HD including somatic, social, and emotional aspects. MATERIALS AND METHODS Of all the patients operated on from 1984 to 1994 at the second hospital of China Medical University for HD, we were able to obtain clearance to recall 76. To assess the long-term outcome and quality of life, 45 patients included in this report were defined as follows: (1) The patients were from 8 to 16 years old. (2) All patients underwent a modified Swenson procedure. (3) HD was confirmed by an operative rectosigmoid biopsy. (4) All patients had an aganglionic segment confined to the rectosigmoid area. (5) Patients excluded from this report were those who had Down’s syndrome or were of subnormal intelligence. Ethical approval was obtained. There were 37 boys and 8 girls. The mean age at the time of Journal of Pediatric Surgery, Vol 37, No 4 (April), 2002: pp 639-642
were significantly lower than those of the controls (11.6 ⫾ 0.7; P ⬍ .05). And the scores of patients who had fecal soiling and incontinence (6.3 ⫾ 2.7) were significantly lower than those of patients without fecal soiling and incontinence (8.4 ⫾ 2.6; P ⬍ .05). Conclusions: Although most patients had good or fair quality of life after surgical correction for HD, the long-term outcome and quality of life are not as good as surgeons expected. The bowel function and quality of life of the patients were poorer than those of healthy children. Fecal soiling is very common and affects patients’ quality of life. Long-term regular follow-up is indispensable. Close attention should be paid to minimizing bowel dysfunction for patients with HD postoperatively to improve their quality of life. J Pediatr Surg 37:639-642. Copyright 2002, Elsevier Science (USA). All rights reserved. INDEX WORDS: Hirschsprung’s disease, long-term follow up, quality of life, fecal soiling.
operation was 19 months (range, 2.5 to 60). The mean follow-up time after operation was 9.1 years (range, 4 to 15.5). The mean age of all patients at time of examination for this study was 10.9 years (range, 8 to 16). Clinical information about previous treatment was taken from the medical records. Families were invited by letter to participate. The patients and their parents were interviewed by a detailed questionnaire. Questions were asked on stool frequency, stool consistency, stool control, medication use, fecal soiling, incontinence, constipation, urinary symptoms, and enterocolitis. Long-term outcome was evaluated by a clinical bowel function scoring system (Table 1). The clinical examination (including a digital rectal examination) was performed by a pediatric surgeon. The definition of constipation used was according to the criteria proposed by Drossman et al.17 Constipation was defined as straining at the stools more than 25% of the time or less than 3 stools per week. Fecal soiling was defined as small amounts of feces having to be scraped off underclothing and at least once a month.6 Incontinence was defined as inability to control the stools more than 1 time per week. Enterocolitis was defined as the presence of abdominal distension, diarrhea or bloody stools, vomiting, and fever.
From the Department of Pediatric Surgery, The Second Clinical College, China Medical University, Shenyang, China. Address reprint requests to WeiLin Wang, MD, The Second Clinical College, China Medical University, No. 36, Sanhao St, Heping District, Shenyang, 110004, China. Copyright 2002, Elsevier Science (USA). All rights reserved. 0022-3468/02/3704-0016$35.00/0 doi:10.1053/jpsu.2002.31625 639
640
BAI ET AL
Table 1. Clinical Bowel Function Scoring System Item
Frequency of defecation
Soiling
Incontinent Fecal sensation
Pain or difficulty with defecation
Fecal consistency
Late Complications
Criteria
Score
Every 2 d or 1-2/d 3-5/d or 2 to 3/wk ⱖ1/wk or ⬎5/d Absent Accidental Frequent Accidental Frequent Normal Defective Missing Never Accidental Frequent Normal Loose Liquid
2 1 0 4 3 2 1 0 2 1 0 2 1 0 2 1 0
NOTE. Good, 9 to 12 points; fair, 5 to 8 points; poor, 0 to 4 points.
Quality of life included inquiry into the patients’ diet, daily life, school absence, relationship to peers, and emotional problems. Quality of life was assessed according to the Quality-of-Life Scoring Criteria for children with fecal incontinence (aged 8 to 16 years old) in a previous study (Table 2).18 Forty-four healthy children without a history of any anorectal surgery and similar age and sex distribution to the patients were used as controls. All data were reported as mean ⫾ SD throughout. Statistical analysis was performed using Student’s t test. Results were considered statistically significant if P value was less than .05.
RESULTS
Stool Control Of 45 patients, 23 patients (51.1%) had long-term bowel dysfunction. Among these, 17 patients (37.8%) had fecal soiling, particularly at night. Three patients (6.7%) suffered from fecal incontinence. Three patients (6.7%) had constipation.
Postoperative enterocolitis occurred in 17 patients (37.8%), 2 patients were found in anastomotic stricture and had obstructive symptoms. Micturition disturbance were observed in 4 patients (8.9%), including acraturesis (1 patient), urinary stuttering (1 patient), and frequency of micturition (2 patients). According to the clinical bowel function scoring system, the scores of patients (7.6 ⫾ 2.1) were significantly lower than those of controls (11.4 ⫾ 0.6; P ⬍ .05). Twenty-one patients (46.7%) had good anal function. Twenty-one patients had fair anal function, and 3 (6.7%) had poor anal function. Quality of Life Because of fecal soiling or incontinence, 25 patients (55.7%) had to restrict their foods. School absence occurred in 6 patients (13.3%). Seven patients (15.6%) had problems in peer relationships. Six patients remain therapy dependent, and 16 (35.6%) patients need occasional intermittent therapy, such as medication, enema, and diapers. According to Quality-of-Life Scoring Criteria for children with fecal incontinence (aged 8 to 16 years), the scores of patients (7.7 ⫾ 2.9) were significantly lower than those of controls (11.6 ⫾ 0.7; P ⬍ .05). There was a strong association between fecal continence and quality of life in patients. The scores of patients who had fecal soiling and incontinence (6.2 ⫾ 2.3) were significantly lower than those of patients without fecal soiling and incontinence (8.6 ⫾ 2.1; P ⬍ .05). Eighteen patients (40%) had good quality of life. Twenty-one patients (46.7%) had fair quality of life, and 6 (13.3%) had poor quality of life. Table 2. Quality of Life Scoring Criteria for Children (Aged 8 to 16 Years) With Fecal Incontinence Item
Stool Frequency Twenty-five patients (55.6%) had 1 or 2 bowel movements per day. Eleven patients (24.4%) had frequency of stooling ranging between 3 and 5 bowel movements per day. Three patients had 1 or 2 bowel movements per week. Six (13.3%) patients had increased frequency of stooling more than 5 times per day (including 3 patients who suffered from fecal incontinence).
Soiling
Incontinence School absence
Unhappy or anxious
Food restriction
Stool Consistency Seven patients (15.7%) had a normal stool consistency. Thirty-five patients (77.8%) had muddy foulsmelling stools and frequent flatus. Three patients sometimes had pasty stools and sometimes had hard stools.
Peer rejection
Criteria
Points
Absent Accidental Frequent Accidental Frequent Never Accidental Frequent Never Accidental Frequent No Somewhat Much Never Accidental Frequent
4 3 2 1 0 2 1 0 2 1 0 2 1 0 2 1 0
NOTE. Good, 9 to 12 points; fair, 5 to 8 points; poor, 0 to 4 points.
QUALITY OF LIFE IN HD
641
DISCUSSION
The functional outcome in patients treated for HD is variable markedly. Many reports showed that the results of surgery for HD were satisfactory. Sherman et al1 reported outcomes with 880 Swenson procedures. At 5 years’ or more follow-up, 89.9% of patients had normal bowel habits; this improved to 93.7% at more than 20 years’ follow-up. In a large review by Bourdelat et al,2 of 2,430 patients receiving a Duhamel operation, at 15 to 30 years’ follow up, the majority of patients achieved good or excellent anal function, only 8.07% patients had constipation, and 5.3% had soiling. However, some studies have indicated less favorable findings. Generally, the postoperative bowel dysfunction had been reported to occur in 10% to 30% of patients with HD. But Heji et al7 reported that about 80% of patients had bowel dysfunction after surgical correction for HD. In our study, 51.1% patients had long-term bowel dysfunction. This may not represent the total experience with HD in our hospital because the patients in this report were a select group. In addition, patients with good functional results often were lost to follow-up. The reason the results were markedly variable is that in the patients in these cases, follow-up time and operation type were different in different reports. In addition, the definitions of soiling, constipation, and incontinence in different studies also were different. In recent years, studies have been published that draw attention to fecal soiling after surgical correction for HD. As was most apparent in this study, the greatest problem in patients after surgery for HD was not constipation, but fecal soiling at long-term follow-up. Fecal soiling is a common and bothersome complication of surgery for HD, occurring in as many as 32% to 80% of patients.13,6 Marty et al13 found that 32% of patients had difficulty with fecal soiling postoperatively, and 12.6% identified this as a severe problem. Catto-Smith et al6 reported a surprisingly high proportion (80%) of fecal soiling after surgery for HD and suspected that soiling was markedly underreported. Fecal soiling is a physically, emotionally, and psychologically disabling symptom. It had a signif-
icant impact on patients’ social and emotional development. It influenced the patients’ quality of life because the patients in this report are now of school age.8,9,18 Quality of life remains a difficult concept to evaluate by the physical, psychological, and social well being of the individual. It is a multidimensional construct that can comprehensively reflect the health. Little is known about the quality of life in patients after surgical treatment for HD. In our reports, we found that some patients had to take drugs or restrict their foods to avoid soiling or diarrhea. Some patients became embarrassed when they had fecal soiling, foul-smelling flatus, and muddy stools and had difficulties in peer relationships. School absence occurred because of fecal soiling especially when the patients had diarrhea. Some patients were unhappy and anxious owing to bowel dysfunction. There was a strong association between fecal continence and quality of life in patients.8 It is the fecal soiling that influenced the quality of life of the patients with HD. Generally, although most patients had good or fair quality of life after surgical correction for HD, the long-term outcome and quality of life are not as good as surgeons expected. The bowel function and quality of life of the patients were poorer than those of healthy children. Fecal soiling is very common and affects patients’ quality of life. Surgical technique should be subjected continuously to improvement of minimizing fecal soiling. Close attention should be paid to the poor fecal continence impact on a child’s social life and emotional development. Moreover, one other point deserving recognition is that, currently, the purpose of the management of HD is not only to ensure survival, but also to achieve normal bowel function and, more importantly, to achieve good quality of life in all possible ways. Surgery is not the end of the treatment. We feel it is necessary to emphasize the long-term regular follow-up of patients after surgery for HD because it is the prerequisite for improvement of the quality of life. Strategies, including psychological counseling,19 medication therapy, and toilet training, are needed.
REFERENCES 1. Sherman J, Snyder M, Weitzman JJ, et al: A 40-year multinational retrospective study of 880 Swenson procedures. J Pediatr Surg 24:833-838, 1989 2. Bourdelat D, Vrsansky P, Page´s R, et al: Duhamel operation 40 years after: A multicentric study. Eur J Pediatr Surg 7:70-76, 1997 3. Fuchs O, Booss D: Rehbein’s procedure for Hirschsprung’s disease. An appraisal of 45 years. Eur J Pediatr Surg 9:389-391, 1999 4. Suita S, Taguchi T, Yanai K, et al: Long-term outcomes and quality of life after Z-shaped anastomosis for Hirschsprung’s disease. J Am Coll Surg 187:577-583, 1998 5. Rescorla FJ, Morrison AM, Engles D, et al: Hirschsprung’s disease: Evaluation of mortality and long-term function in 260 cases. Arch Surg 127:934-942, 1992 6. Catto-Smith AG, Coffey CMM, Nolan TM, et al: Fecal inconti-
nence after the surgical treatment of Hirschsprung’s disease. J Pediatr 127:954-957, 1995 7. Heij HA, de Vries X, Bremer I, et al: Long-term anorectal function after Duhamel operation for Hirschsprung’s disease. J Pediatr Surg 30:430-432, 1995 8. Yanchar NL, Soucy P: Long-term outcome after Hirschsprung’s disease: Patients’ perspectives. J Pediatr Surg 34:1152-1160, 1999 9. Moore SW, Albertyn R, Cywes S: Clinical outcome and longterm quality of life after surgical correction of Hirschsprung’s disease. J Pediatr Surg 31:1496-1502, 1996 10. Reding R, de Ville de Goyet J, Gosseye S, et al: Hirschsprung’s disease: A 20-year experience. J Pediatr Surg 32:1221-1225, 1997 11. Heikkinen M, Rintala R, Luukkonen P: Long-term anal sphinc-
642
ter performance after surgery for Hirschsprung’s disease. J Pediatr Surg 32:1443-1446, 1997 12. Fortuna RS, Weber TR, Tracy TF, et al: Critical analysis of the operative treatment of Hirschsprung’s disease. Arch Surg 131:520-525, 1996 13. Marty TL, Seo T, Matlak ME, et al: Gastrointestinal function after surgical correction of Hirschsprung’s disease: Long-term follow-up in 135 patients. J Pediatr Surg 30:655-658, 1995 14. Mishalany HG, Woolley MM: Postoperative functional and manometric evaluation of patients with Hirschsprung’s disease. J Pediatr Surg 22:443-446, 1987 15. Bjornland K, Diseth TH, Emblem R: Long-term functional, manometric, and endosonographic evaluation of patients operated upon with the Duhamel technique. Pediatr Surg Int 13:24-28, 1998
BAI ET AL
16. Nagasaki A, Ikeda K, Suita S: Postoperative sequential anorectal manometric study of children with Hirschsprung’s disease. J Pediatr Surg 15:615-619, 1980 17. Drossman DA, Sandler RS, Mckee DC, et al: Bowel patterns among subjects not seeking health care. Use of a questionnaire to identify a population with bowel dysfunction. Gastroenterology 83: 529-534, 1982 18. Bai Yuzuo, Yuan Zhengwei, Wang Weilin, et al: Quality of life for children with fecal incontinence after surgically corrected anorectal malformation. J Pediatr Surg 35:462-464, 2000 19. Diseth TH, Bjornland K, Novik TS, et al: Bowel function, mental health, and psychosocial function in adolescents with Hirschsprung’s disease. Arch Dis Child 76:100-106, 1997
Background/Purpose: The aim of this study was to investigate long-term outcome and quality of life after the Swenson operation for rectosigmoid Hirschsprung’s Disease (HD). Methods: Forty-five patients who underwent the Swenson procedure for HD underwent follow-up for 8 to 16 years. Long-term outcome and quality of life were assessed by interviews and questionnaires including scoring systems. Forty-four healthy children with similar age, sex, and education level distributions used as controls. Results: In 45 patients, 23 (51.1%) had bowel dysfunction. Seventeen patients (37.8%) suffered from fecal soiling. According to the clinical bowel function scoring system, the patients’ scores (7.6 ⫾ 2.1) were significantly lower than those of the controls (11.4 ⫾ 0.6; P ⬍ .05). Because of poor fecal continence, 25 patients (55.7%) had to restrict their foods. School absence occurred in 6 (13.3%) patients. Seven patients (15.6%) had problems in peer relationships. According to the Quality-of-Life Scoring Criteria, 86.7% patients had good or fair quality of life. The patients’ scores (7.7 ⫾ 2.9)
W
ITH MANY ADVANCES in the surgical management of Hirschsprung’s disease (HD) in the modern era, the postoperative results of surgically treated HD generally are satisfactory.1-5 However, some patients continued to have persistent bowel dysfunction including incontinence, constipation, and enterocolitis. Several reports showed less favorable findings. The long-term outcome of surgery for HD is variable.1-16 In addition, the majority of long-term follow-up studies have concentrated only on the functional outcome. Although this is important, little is known about the quality of life in patients after surgical treatment for HD. The aim of this study was to investigate the long-term clinical outcome and quality of life in patients with surgical correction of HD including somatic, social, and emotional aspects. MATERIALS AND METHODS Of all the patients operated on from 1984 to 1994 at the second hospital of China Medical University for HD, we were able to obtain clearance to recall 76. To assess the long-term outcome and quality of life, 45 patients included in this report were defined as follows: (1) The patients were from 8 to 16 years old. (2) All patients underwent a modified Swenson procedure. (3) HD was confirmed by an operative rectosigmoid biopsy. (4) All patients had an aganglionic segment confined to the rectosigmoid area. (5) Patients excluded from this report were those who had Down’s syndrome or were of subnormal intelligence. Ethical approval was obtained. There were 37 boys and 8 girls. The mean age at the time of Journal of Pediatric Surgery, Vol 37, No 4 (April), 2002: pp 639-642
were significantly lower than those of the controls (11.6 ⫾ 0.7; P ⬍ .05). And the scores of patients who had fecal soiling and incontinence (6.3 ⫾ 2.7) were significantly lower than those of patients without fecal soiling and incontinence (8.4 ⫾ 2.6; P ⬍ .05). Conclusions: Although most patients had good or fair quality of life after surgical correction for HD, the long-term outcome and quality of life are not as good as surgeons expected. The bowel function and quality of life of the patients were poorer than those of healthy children. Fecal soiling is very common and affects patients’ quality of life. Long-term regular follow-up is indispensable. Close attention should be paid to minimizing bowel dysfunction for patients with HD postoperatively to improve their quality of life. J Pediatr Surg 37:639-642. Copyright 2002, Elsevier Science (USA). All rights reserved. INDEX WORDS: Hirschsprung’s disease, long-term follow up, quality of life, fecal soiling.
operation was 19 months (range, 2.5 to 60). The mean follow-up time after operation was 9.1 years (range, 4 to 15.5). The mean age of all patients at time of examination for this study was 10.9 years (range, 8 to 16). Clinical information about previous treatment was taken from the medical records. Families were invited by letter to participate. The patients and their parents were interviewed by a detailed questionnaire. Questions were asked on stool frequency, stool consistency, stool control, medication use, fecal soiling, incontinence, constipation, urinary symptoms, and enterocolitis. Long-term outcome was evaluated by a clinical bowel function scoring system (Table 1). The clinical examination (including a digital rectal examination) was performed by a pediatric surgeon. The definition of constipation used was according to the criteria proposed by Drossman et al.17 Constipation was defined as straining at the stools more than 25% of the time or less than 3 stools per week. Fecal soiling was defined as small amounts of feces having to be scraped off underclothing and at least once a month.6 Incontinence was defined as inability to control the stools more than 1 time per week. Enterocolitis was defined as the presence of abdominal distension, diarrhea or bloody stools, vomiting, and fever.
From the Department of Pediatric Surgery, The Second Clinical College, China Medical University, Shenyang, China. Address reprint requests to WeiLin Wang, MD, The Second Clinical College, China Medical University, No. 36, Sanhao St, Heping District, Shenyang, 110004, China. Copyright 2002, Elsevier Science (USA). All rights reserved. 0022-3468/02/3704-0016$35.00/0 doi:10.1053/jpsu.2002.31625 639
640
BAI ET AL
Table 1. Clinical Bowel Function Scoring System Item
Frequency of defecation
Soiling
Incontinent Fecal sensation
Pain or difficulty with defecation
Fecal consistency
Late Complications
Criteria
Score
Every 2 d or 1-2/d 3-5/d or 2 to 3/wk ⱖ1/wk or ⬎5/d Absent Accidental Frequent Accidental Frequent Normal Defective Missing Never Accidental Frequent Normal Loose Liquid
2 1 0 4 3 2 1 0 2 1 0 2 1 0 2 1 0
NOTE. Good, 9 to 12 points; fair, 5 to 8 points; poor, 0 to 4 points.
Quality of life included inquiry into the patients’ diet, daily life, school absence, relationship to peers, and emotional problems. Quality of life was assessed according to the Quality-of-Life Scoring Criteria for children with fecal incontinence (aged 8 to 16 years old) in a previous study (Table 2).18 Forty-four healthy children without a history of any anorectal surgery and similar age and sex distribution to the patients were used as controls. All data were reported as mean ⫾ SD throughout. Statistical analysis was performed using Student’s t test. Results were considered statistically significant if P value was less than .05.
RESULTS
Stool Control Of 45 patients, 23 patients (51.1%) had long-term bowel dysfunction. Among these, 17 patients (37.8%) had fecal soiling, particularly at night. Three patients (6.7%) suffered from fecal incontinence. Three patients (6.7%) had constipation.
Postoperative enterocolitis occurred in 17 patients (37.8%), 2 patients were found in anastomotic stricture and had obstructive symptoms. Micturition disturbance were observed in 4 patients (8.9%), including acraturesis (1 patient), urinary stuttering (1 patient), and frequency of micturition (2 patients). According to the clinical bowel function scoring system, the scores of patients (7.6 ⫾ 2.1) were significantly lower than those of controls (11.4 ⫾ 0.6; P ⬍ .05). Twenty-one patients (46.7%) had good anal function. Twenty-one patients had fair anal function, and 3 (6.7%) had poor anal function. Quality of Life Because of fecal soiling or incontinence, 25 patients (55.7%) had to restrict their foods. School absence occurred in 6 patients (13.3%). Seven patients (15.6%) had problems in peer relationships. Six patients remain therapy dependent, and 16 (35.6%) patients need occasional intermittent therapy, such as medication, enema, and diapers. According to Quality-of-Life Scoring Criteria for children with fecal incontinence (aged 8 to 16 years), the scores of patients (7.7 ⫾ 2.9) were significantly lower than those of controls (11.6 ⫾ 0.7; P ⬍ .05). There was a strong association between fecal continence and quality of life in patients. The scores of patients who had fecal soiling and incontinence (6.2 ⫾ 2.3) were significantly lower than those of patients without fecal soiling and incontinence (8.6 ⫾ 2.1; P ⬍ .05). Eighteen patients (40%) had good quality of life. Twenty-one patients (46.7%) had fair quality of life, and 6 (13.3%) had poor quality of life. Table 2. Quality of Life Scoring Criteria for Children (Aged 8 to 16 Years) With Fecal Incontinence Item
Stool Frequency Twenty-five patients (55.6%) had 1 or 2 bowel movements per day. Eleven patients (24.4%) had frequency of stooling ranging between 3 and 5 bowel movements per day. Three patients had 1 or 2 bowel movements per week. Six (13.3%) patients had increased frequency of stooling more than 5 times per day (including 3 patients who suffered from fecal incontinence).
Soiling
Incontinence School absence
Unhappy or anxious
Food restriction
Stool Consistency Seven patients (15.7%) had a normal stool consistency. Thirty-five patients (77.8%) had muddy foulsmelling stools and frequent flatus. Three patients sometimes had pasty stools and sometimes had hard stools.
Peer rejection
Criteria
Points
Absent Accidental Frequent Accidental Frequent Never Accidental Frequent Never Accidental Frequent No Somewhat Much Never Accidental Frequent
4 3 2 1 0 2 1 0 2 1 0 2 1 0 2 1 0
NOTE. Good, 9 to 12 points; fair, 5 to 8 points; poor, 0 to 4 points.
QUALITY OF LIFE IN HD
641
DISCUSSION
The functional outcome in patients treated for HD is variable markedly. Many reports showed that the results of surgery for HD were satisfactory. Sherman et al1 reported outcomes with 880 Swenson procedures. At 5 years’ or more follow-up, 89.9% of patients had normal bowel habits; this improved to 93.7% at more than 20 years’ follow-up. In a large review by Bourdelat et al,2 of 2,430 patients receiving a Duhamel operation, at 15 to 30 years’ follow up, the majority of patients achieved good or excellent anal function, only 8.07% patients had constipation, and 5.3% had soiling. However, some studies have indicated less favorable findings. Generally, the postoperative bowel dysfunction had been reported to occur in 10% to 30% of patients with HD. But Heji et al7 reported that about 80% of patients had bowel dysfunction after surgical correction for HD. In our study, 51.1% patients had long-term bowel dysfunction. This may not represent the total experience with HD in our hospital because the patients in this report were a select group. In addition, patients with good functional results often were lost to follow-up. The reason the results were markedly variable is that in the patients in these cases, follow-up time and operation type were different in different reports. In addition, the definitions of soiling, constipation, and incontinence in different studies also were different. In recent years, studies have been published that draw attention to fecal soiling after surgical correction for HD. As was most apparent in this study, the greatest problem in patients after surgery for HD was not constipation, but fecal soiling at long-term follow-up. Fecal soiling is a common and bothersome complication of surgery for HD, occurring in as many as 32% to 80% of patients.13,6 Marty et al13 found that 32% of patients had difficulty with fecal soiling postoperatively, and 12.6% identified this as a severe problem. Catto-Smith et al6 reported a surprisingly high proportion (80%) of fecal soiling after surgery for HD and suspected that soiling was markedly underreported. Fecal soiling is a physically, emotionally, and psychologically disabling symptom. It had a signif-
icant impact on patients’ social and emotional development. It influenced the patients’ quality of life because the patients in this report are now of school age.8,9,18 Quality of life remains a difficult concept to evaluate by the physical, psychological, and social well being of the individual. It is a multidimensional construct that can comprehensively reflect the health. Little is known about the quality of life in patients after surgical treatment for HD. In our reports, we found that some patients had to take drugs or restrict their foods to avoid soiling or diarrhea. Some patients became embarrassed when they had fecal soiling, foul-smelling flatus, and muddy stools and had difficulties in peer relationships. School absence occurred because of fecal soiling especially when the patients had diarrhea. Some patients were unhappy and anxious owing to bowel dysfunction. There was a strong association between fecal continence and quality of life in patients.8 It is the fecal soiling that influenced the quality of life of the patients with HD. Generally, although most patients had good or fair quality of life after surgical correction for HD, the long-term outcome and quality of life are not as good as surgeons expected. The bowel function and quality of life of the patients were poorer than those of healthy children. Fecal soiling is very common and affects patients’ quality of life. Surgical technique should be subjected continuously to improvement of minimizing fecal soiling. Close attention should be paid to the poor fecal continence impact on a child’s social life and emotional development. Moreover, one other point deserving recognition is that, currently, the purpose of the management of HD is not only to ensure survival, but also to achieve normal bowel function and, more importantly, to achieve good quality of life in all possible ways. Surgery is not the end of the treatment. We feel it is necessary to emphasize the long-term regular follow-up of patients after surgery for HD because it is the prerequisite for improvement of the quality of life. Strategies, including psychological counseling,19 medication therapy, and toilet training, are needed.
REFERENCES 1. Sherman J, Snyder M, Weitzman JJ, et al: A 40-year multinational retrospective study of 880 Swenson procedures. J Pediatr Surg 24:833-838, 1989 2. Bourdelat D, Vrsansky P, Page´s R, et al: Duhamel operation 40 years after: A multicentric study. Eur J Pediatr Surg 7:70-76, 1997 3. Fuchs O, Booss D: Rehbein’s procedure for Hirschsprung’s disease. An appraisal of 45 years. Eur J Pediatr Surg 9:389-391, 1999 4. Suita S, Taguchi T, Yanai K, et al: Long-term outcomes and quality of life after Z-shaped anastomosis for Hirschsprung’s disease. J Am Coll Surg 187:577-583, 1998 5. Rescorla FJ, Morrison AM, Engles D, et al: Hirschsprung’s disease: Evaluation of mortality and long-term function in 260 cases. Arch Surg 127:934-942, 1992 6. Catto-Smith AG, Coffey CMM, Nolan TM, et al: Fecal inconti-
nence after the surgical treatment of Hirschsprung’s disease. J Pediatr 127:954-957, 1995 7. Heij HA, de Vries X, Bremer I, et al: Long-term anorectal function after Duhamel operation for Hirschsprung’s disease. J Pediatr Surg 30:430-432, 1995 8. Yanchar NL, Soucy P: Long-term outcome after Hirschsprung’s disease: Patients’ perspectives. J Pediatr Surg 34:1152-1160, 1999 9. Moore SW, Albertyn R, Cywes S: Clinical outcome and longterm quality of life after surgical correction of Hirschsprung’s disease. J Pediatr Surg 31:1496-1502, 1996 10. Reding R, de Ville de Goyet J, Gosseye S, et al: Hirschsprung’s disease: A 20-year experience. J Pediatr Surg 32:1221-1225, 1997 11. Heikkinen M, Rintala R, Luukkonen P: Long-term anal sphinc-
642
ter performance after surgery for Hirschsprung’s disease. J Pediatr Surg 32:1443-1446, 1997 12. Fortuna RS, Weber TR, Tracy TF, et al: Critical analysis of the operative treatment of Hirschsprung’s disease. Arch Surg 131:520-525, 1996 13. Marty TL, Seo T, Matlak ME, et al: Gastrointestinal function after surgical correction of Hirschsprung’s disease: Long-term follow-up in 135 patients. J Pediatr Surg 30:655-658, 1995 14. Mishalany HG, Woolley MM: Postoperative functional and manometric evaluation of patients with Hirschsprung’s disease. J Pediatr Surg 22:443-446, 1987 15. Bjornland K, Diseth TH, Emblem R: Long-term functional, manometric, and endosonographic evaluation of patients operated upon with the Duhamel technique. Pediatr Surg Int 13:24-28, 1998
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